Lymphoma
Lymphoma
Classification and external resources |
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Gastric MALT lymphoma |
ICD-10 |
C81.-C96. |
ICD-O: |
9590-9999 |
MeSH |
D008223 |
Lymphoma a type of neoplasm that originates in lymphocytes (a type of white blood cell in the vertebrate immune system). They often originate in lymph nodes, presenting as an enlargement of the node (a tumor). Lymphomas are closely related to lymphoid leukemias, which also originate in lymphocytes but do not form tumors.[1] There are many types of lymphomas, and in turn, lymphomas are a part of the broad group of diseases called hematological neoplasms.
Thomas Hodgkin published in 1832 the first description of lymphoma, specifically of the form named after him, Hodgkin's lymphoma. Since, many other forms of lymphoma have been described and many classifications proposed. The 1982 Working formulation classification became very popular. It introduced the category Non-Hodgkin lymphoma (NHL), itself divided into 16 different diseases. However, since these different lymphomas have little in common with each other, the NHL label is of limited usefulness for doctors or patients and is slowly being abandoned. The latest classification by the WHO (2001) lists 43 different forms of lymphoma divided in four broad groups.
Some forms of Lymphoma are indolent (e.g. Small lymphocytic lymphoma), compatible with a long life even without treatment, whereas other forms are aggressive (e.g. Burkitt's lymphoma), causing rapid deterioration and death. The prognosis therefore depends on the correct classification of the disease, established by a pathologist after examination of a biopsy. Chemotherapy is prescribed by an oncologist, radiation therapy by a radiation oncologist.
Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B, T or NK cell lineage. True Histiocytic malignancies are rare and are classified as sarcomas.[2]
Prevalence
According to the U.S. National Institutes of Health, lymphomas account for about five percent of all cases of cancer in the United States, and Hodgkin's lymphoma in particular accounts for less than one percent of all cases of cancer in the United States.
Because the whole system is part of the body's immune system, patients with a weakened immune system, such as from HIV infection or from certain drugs or medication, also have a higher incidence of lymphoma.
Classification
REAL classification
In the 1990s, the Revised European-American Lymphoma (REAL) Classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic NHL entities. [3]
WHO classification
The WHO Classification, published in 2001 and updated in 2008[2], is the latest classification of lymphoma and is based on the "Revised European-American Lymphoma classification" (REAL). This system attempts to group lymphomas by cell type, i.e., the normal cell type that most resembles the tumor. There are three large groups: the B cell, T cell, and natural killer cell tumors. Other less common groups, including Hodgkin's lymphoma, are also recognized. (ICD-O codes are provided where available.)
Mature B cell neoplasms
DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. Colors indicate levels of expression; green indicates genes that are underexpressed in lymphoma cells (as compared to normal cells), whereas red indicates genes that are overexpressed in lymphoma cells.
- Chronic lymphocytic leukemia/Small lymphocytic lymphoma
- B-cell prolymphocytic leukemia
- Lymphoplasmacytic lymphoma (such as Waldenström macroglobulinemia)
- Splenic marginal zone lymphoma
- Plasma cell neoplasms:
- Plasma cell myeloma
- Plasmacytoma
- Monoclonal immunoglobulin deposition diseases
- Heavy chain diseases
- Extranodal marginal zone B cell lymphoma, also called MALT lymphoma
- Nodal marginal zone B cell lymphoma (NMZL)
- Follicular lymphoma
- Mantle cell lymphoma
- Diffuse large B cell lymphoma
- Mediastinal (thymic) large B cell lymphoma
- Intravascular large B cell lymphoma
- Primary effusion lymphoma
- Burkitt lymphoma/leukemia
Mature T cell and natural killer (NK) cell neoplasms
- T cell prolymphocytic leukemia
- T cell large granular lymphocytic leukemia
- Aggressive NK cell leukemia
- Adult T cell leukemia/lymphoma
- Extranodal NK/T cell lymphoma, nasal type
- Enteropathy-type T cell lymphoma
- Hepatosplenic T cell lymphoma
- Blastic NK cell lymphoma
- Mycosis fungoides / Sezary syndrome
- Primary cutaneous CD30-positive T cell lymphoproliferative disorders
- Primary cutaneous anaplastic large cell lymphoma
- Lymphomatoid papulosis
- Angioimmunoblastic T cell lymphoma
- Peripheral T cell lymphoma, unspecified
- Anaplastic large cell lymphoma
Hodgkin lymphoma
- Classical Hodgkin lymphomas:
- Nodular sclerosis
- Mixed cellularity
- Lymphocyte-rich
- Lymphocyte depleted or not depleted
- Nodular lymphocyte-predominant Hodgkin lymphoma
Immunodeficiency-associated lymphoproliferative disorders
- Associated with a primary immune disorder
- Associated with the Human Immunodeficiency Virus (HIV)
- Post-transplant
- Associated with Methotrexate therapy
Working formulation and Non-Hodgkin lymphoma
The 1982 Working Formulation is a classification of Non-Hodgkin Lymphoma. It has since been replaced by other lymphoma classifications, the latest published by the WHO in 2001, but is still used by cancer agencies for compilation of lymphoma statistics.
Other classification systems
- ICD-O (codes 9590-9999, details at [1]) (archive link, was dead)
- ICD-10 (codes C81-C96, details at [2])
Diagnosis, etiology, staging, prognosis, and treatment
See the article of the corresponding form of lymphoma.
See also
- Hodgkin's lymphoma
- Non-Hodgkin's lymphoma
- Follicular lymphoma
- Burkitt's lymphoma
- Mantle cell lymphoma
- Gastric lymphoma
- Cutaneous T Cell lymphoma
- Mycosis fungoides
- Anaplastic large cell lymphoma
- MALT lymphoma
- Primary central nervous system lymphoma
- BCP-1 cells
- Ann Arbor staging
- International Prognostic Index
References
- ↑ Parham, Peter (2005). The immune system. New York: Garland Science. pp. 414. ISBN 0-8153-4093-1.
- ↑ 2.0 2.1 Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6.
- ↑ www.emedicine.com on Lymphoma, Non-Hodgkin
External links
Pathology: hematology · myeloid hematologic disease (primarily D50-D77 · 280-289) |
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RBCs/
hemoglobinopathy |
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Polycythemia · Macrocytosis
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Nutritional
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Iron deficiency anemia (Plummer-Vinson syndrome) · Megaloblastic anemia ( Pernicious anemia)
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Hemolytic
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Hereditary
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enzyme: G6PD Deficiency · Pyruvate kinase deficiency · Triosephosphate isomerase deficiency
hemoglobin: Thalassemia · Sickle-cell disease/trait
membrane: Hereditary spherocytosis · Hereditary elliptocytosis · Hereditary stomatocytosis
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Acquired
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Autoimmune (Warm, Cold) · MAHA · Myelophthisic
combinations: HUS · hemoglobinuria (PNH, PCH)
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Aplastic
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Acquired PRCA · Diamond-Blackfan anemia · Fanconi anemia · Sideroblastic anemia
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Blood tests
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MCV (Normocytic, Microcytic, Macrocytic) · MCHC (Normochromic, Hypochromic)
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Other
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Methemoglobinemia
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Coagulation/platelets/
coagulopathy/
bleeding diathesis |
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Hypercoagulability
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primary: Antithrombin III deficiency · Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden · Hyperprothrombinemia
acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) · autoimmune ( Antiphospholipid)
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Other
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Essential thrombocytosis
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clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II · XIII
platelet function: Bernard-Soulier syndrome · Glanzmann's thrombasthenia · Hermansky-Pudlak syndrome · Gray platelet syndrome · May Hegglin anomaly · Pelger-Huet anomaly
Purpura: Henoch-Schönlein · TP · ITP (Evans syndrome) · TTP
Thrombocytopenia (Heparin-induced thrombocytopenia)
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Monocytes/
macrophages |
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Histiocytosis
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WHO-I (Langerhans cell histiocytosis)
WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis)
WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
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Other
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-penia: Monocytopenia
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Granulocytes |
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-cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia)
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See also hematological malignancy and immune disorders
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Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590-9739, 9800-9839) |
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B cell
(lymphoma,
leukemia)
(most CD19, CD20)
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TdT+
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ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
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CD5+
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SLL/ CLL - mantle zone (Mantle cell)
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CD22+
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Prolymphocytic - Hairy cell leukemia
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CD79a+
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marginal zone (Splenic marginal zone, MALT, Nodal marginal zone)
germinal center (Follicular, Burkitt's, Diffuse large)
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Other B
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Primary effusion - Lymphomatoid granulomatosis
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Plasma cell dyscrasias
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Plasmacytoma - Multiple myeloma - Waldenström's macroglobulinemia - Heavy chain disease - MGUS - Primary amyloidosis —- Plasma cell leukemia
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TdT+
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ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
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TdT-
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Cutaneous - Mycosis fungoides/Sézary's disease, Angioimmunoblastic - Anaplastic large cell - Hepatosplenic - Lymphomatoid papulosis
Adult - Large granular lymphocyte - Prolymphocytic - Acute lymphoblastic
Aggressive NK-cell leukemia
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Other
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See also lymphatic disease
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